[Perivascular epithelioid cell tumours of the uterus]

Perivascular epithelioid cell tumours (PEComas) are rare tumours with unknown malignant potential. We report a case of a 48-year-old woman, who had had many tumours, initially diagnosed as leiomyomas, removed from her uterus and vagina over 16 years. A recurrent tumour and revision of prior specimens revealed PEComa. As free surgical margins could not be obtained, the woman is regularly controlled by magnetic resonance imaging. So far recurrence has not been detected. This case shows that knowledge of PEComas is important to ensure relevant follow-up as these are low malignant tumours that tend to reoccur.